title: Effects of long-term sildenafil treatment for pulmonary hypertension in infants with chronic lung disease.
abstract: OBJECTIVE
  To determine the clinical course and outcomes of infants with chronic lung disease (CLD) and pulmonary hypertension (PH) who received prolonged sildenafil therapy.
  
  
  METHODS
  We conducted a retrospective review of 25 patients <2 years of age with CLD in whom sildenafil was initiated for the treatment of PH while they were hospitalized from January 2004 to October 2007. Hemodynamic improvement was defined by a 20% decrease in the ratio of pulmonary to systemic systolic arterial pressure or improvement in the degree of ventricular septal flattening with serial echocardiograms.
  
  
  RESULTS
  Chronic sildenafil therapy (dose range, 1.5-8.0 mg/kg/d) was initiated at a median of 171 days of age (range, 14-673 days of age) for a median duration of 241 days (range, 28-950 days). Twenty-two patients (88%) achieved hemodynamic improvement after a median treatment duration of 40 days (range, 6-600 days). Eleven of the 13 patients with interval estimates of systolic pulmonary artery pressure with echocardiogram showed clinically significant reductions in PH. Five patients (20%) died during the follow-up period. Adverse events leading to cessation or interruption of therapy occurred in 2 patients, 1 for recurrent erections, and the other had the medication held briefly because of intestinal pneumatosis.
  
  
  CONCLUSIONS
  These data suggest that chronic sildenafil therapy is well-tolerated, safe, and effective for infants with PH and CLD.
authors.affiliation: Division of Critical Care, The Children's Hospital and University of Colorado Denver, School of Medicine, Aurora, CO, USA.
authors.forename: Peter M
authors.initials: PM
authors.lastname: Mourani
authors.forename: Marci K
authors.initials: MK
authors.lastname: Sontag
authors.forename: D Dunbar
authors.initials: DD
authors.lastname: Ivy
authors.forename: Steven H
authors.initials: SH
authors.lastname: Abman
mesh_terms: D001794:Blood Pressure / Q000187:drug effects; D001997:Bronchopulmonary Dysplasia / Q000150:complications*; D004452:Echocardiography; D005260:Female; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D008297:Male; D010726:Phosphodiesterase Inhibitors / Q000008:administration & dosage* / Q000009:adverse effects; D010879:Piperazines / Q000008:administration & dosage* / Q000009:adverse effects; D011651:Pulmonary Artery / Q000187:drug effects; D011687:Purines / Q000008:administration & dosage / Q000009:adverse effects; D012189:Retrospective Studies; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage* / Q000009:adverse effects; D016896:Treatment Outcome
chemical_list: D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D000068677:Sildenafil Citrate